Relationship between L-valine catabolism and S-β-aminoisobutyrate (S-β-AiB) metabolism. The circled numbers indicate the following enzymes: 1 = branched-chain.

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Relationship between L-valine catabolism and S-β-aminoisobutyrate (S-β-AiB) metabolism. The circled numbers indicate the following enzymes: 1 = branched-chain amino acid transaminase(s); 2 = branched-chain α-keto acid dehydrogenase; 3 = isobutyryl-CoA dehydrogenase; 4 = enoyl-CoA hydratase; 5 = 3-hydroxyisobutyryl-CoA hydrolase; 6 = an acyl-CoA synthetase (?); 7 = 3-hydroxyisobutyrate dehydrogenase; 8 = methylmalonate semialdehyde dehydrogenase (acylating); 9 = propionyl-CoA carboxylase; 10 = methylmalonyl-CoA racemase; 11 = methylmalonyl-CoA mutase; 12 = thioester hydrolase (?); 13 = S-β-aminoisobutyrate-α-ketoglutarate transaminase. Disorders of valine oxidation (A) (Gibson et al. 1999, 102, 250, 105). may affect reaction 13. step. (Pathway diagram reproduced from Griffith, (Adam et al. 2) with permission.) Source: Acknowledgments: Disorders of β- and γ-Amino Acids in Free and Peptide-Linked Forms The authors have drawn substantively from the earlier version of this chapter (MMBID 7th Edition, Vol 1, Chapter 38, pp. 1349–1368) and gratefully acknowledge the significant contribution of the preceding primary author, Dr. Charles R. Scriver. , The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: https://ommbid.mhmedical.com/DownloadImage.aspx?image=/data/Books/971/ch91fg5.png&sec=62676526&BookID=971&ChapterSecID=62676426&imagename= Accessed: October 14, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved